The biochemistry Core Laboratory (Project IB) will provide the central biochemistry facility for the program project. Its investigators will participate in patient recruitment and will provide the following specific laboratory services and studies: 1. Patient sample collection and preparation including: tissue culture; leukocyte, urine, plasma and red blood cell preparation; sample storage and distribution. 2. Acyl-CoA dehydrogenase assays on fibroblasts, leukocytes, amniotic fluid cells, and other tissues. 3. Assays of fatty acid oxidation in fibroblasts. 4. Gas chromatography-mass spectrometry analysis of urine and plasma metabolites. The Biochemistry Core Laboratory investigators will work jointly with the Clinical Core investigators to ensure timely collection and interpretation of patient-related data obtained from these studies.